Neurología : publicación oficial de la Sociedad Española de Neurología
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Spontaneous intracranial hypotension (SIH) is characterized by orthostatic headache and diffuse dural gadolinium enhancement on magnetic resonance imaging. Spontaneous intracranial hypotension is caused by spinal cerebrospinal fluid leaks and evidence for an underlying generalized connective tissue disorder is found in about two thirds of patients. There had been no familial cases reported to date. ⋯ These familial cases of spontaneous SIH suggest that certain underlying genetic susceptibility, probably linked to lower resistance of the spinal meninges, could favor the development of spontaneous intracranial hypotension.